Clearance of Toxic Proteins from the CSF
ALS & Frontal Temporal Dementia
TDP-43 & DPRs
Accumulation of the TDP43 protein and Dipeptide Repeat proteins (DPRs) have been shown to drive neurodegeneration in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). TDP43 and DPRs pathology occurs in the majority of ALS cases and the abnormal form of these proteins is present in the cerebrospinal fluid (CSF) which bathes the nervous system. The flow of CSF distributes the toxic forms of these proteins throughout the brain and spinal cord. Motor neurons which are the largest neurons in the central nervous system and control bodily movement are exquisitely sensitive to pathological TDP43 and DPRs. The entry of TDP43 and DPRs into these neurons causes cell death which progressively leads to paralysis and eventually the tragic inability to breathe.
EnClear has developed a proprietary technology which clears TDP43 and DPRs from the CSF. We are currently developing this technology into a device that will circulate the CSF and significantly reduce or eliminate pathological TDP43/DPR. With this technology EnClear aims to stop or significantly slow the neuronal degeneration in ALS and FTD patients. This technology can potentially be used for other toxic proteins which are known to spread in other neurodegenerative disorders.*
Progressive Supranuclear Palsy
Similar to ALS, Progressive Supranuclear Palsy (PSP) is hallmarked by the buildup of toxic proteins (TAU) in the brain. As with ALS, the toxic proteins travel through the brain and spinal fluids and are taken up by neurons that are very sensitive to their toxicity which leads to degeneration of these neurons leading to paralysis. EnClear Therapies aims to halt the progression of PSP by rendering the toxic proteins (TAU) harmless through the same mechanism used for ALS.
Approximately 20,000 Americans have PSP (~3-6 in 100,000 people worldwide). The average age of diagnosis for PSP is 60. These patents have a 2-10 year life expectancy post-diagnosis with common complications including pneumonia, choking, head injury, fractures and eventual death.
* The EnClear platform is in early development, statements are currently under investigation.